Calvin & Teddy's Medical Journey
Our Story has unfolded significantly since we first started our GofundMe. While, the story unfolded, as stories do, from a beginning that seemed certain to a more unexpected turn of events, please read the updates to know the most current update for the boys. Right now, Calvin goes for Intravenous Immunoglobulin Infusions (IVIG) ever three weeks. Each infusion is hours long and we head to Children's when I get off of work from 4:00pm-9:00pm, which is exhausting, but he is so positive and strong. These infusions battle his primary immunodeficiency disorder, "Common Variable Immune Deficiency", "Hypogammaglobulimia", and "Specific Antibody Deficiency." Doctors have gone back and forth on whether or not Calvin also has a incurable lung disease known as, Primary Ciliary Dyskinesia, but we are STILL in the process of figuring that out as Teddy came back as a definite disease carrier and with Calvin's presentation, they are retesting him as it is likely he has this disease as all doctor previously diagnosed based on his clinical symptoms and genetic results. Calvin also suffers from Idiopathic Anaphylaxis, but we seemingly have had that under control as of recent.
If you have read the updates, many of you know that Teddy is not doing as well as Calvin. The list of his struggles are too long to discuss at this time, but we are currently dealing with bone marrow dysfunction, which is significant as he came back positive for a genetic mutation of VUS known as SAMD9L on a bone marrow failure panel, which predisposes him to a condition known as Monosomy 7 MDS, which has a very poor prognosis. His marrow was normal last year, so initially, doctors thought there was a chance he might not develop marrow dysfunction, however, last month, his pathology report showed he likely has MDS as his results showed hypocellular marrow changes and dysmyelopoiesis. Inherited Pediatric MDS is extremely rare and poorly understood. It is often fatal. We don't know much about Teddy's prognosis other than his pathology was abnormal last month and that he was immediately accepted into a clinical trial at St. Jude for inherited pediatric MDS. Teddy either has MDS with high risk for Acute Myeloid Leukemia with poor prognosis, or by some miracle, could have a less extreme condition that is affecting his marrow. Nevertheless, while less severe and not fatal, is still serious. He is clinically very symptomatic and the only cure is a Bone Marrow Transplant if St. Jude concludes he is in fact in bone marrow failure. We won't know any confirmed type of diagnosis or confirmation of type of MDS until he has a repeat (third) marrow biopsy in December and goes to St. Jude at the end of September. St. Jude will be guiding his treatment plan and Dana Farber's Jimmy Fund Clinic will be carrying out most of his treatment here in Boston, whatever that may be.
Sadly, we also found out Calvin will need to be a patient at St. Jude too as the team wants him to be tested for the bone marrow failure to see if he has Teddy's mutation and needs monitoring or a marrow biopsy. This breaks my heart, but I know we have the best of the best looking after my boys. It's just hard because my mother has Lymphoma and Breast Cancer, and my boys are so ill. They are fighters though. And inspire me constantly with their positivity.
Teddy also battles intestinal malabsorption, pancreatic insufficiency, failure to thrive, skeletal dysplasia, severe hypoglycemia that has led to metabolic crisis (where we almost lost him last month the hospital), muscle weakness/low tone/poor balance/poor endurance, (requiring weekly PT and bilateral leg braces), and most likely a wheelchair for school. He suffers from epilepsy, is back on his G-tube, and suffers numerous infections and constant fevers, including a ten week fever just months ago, despite being in isolation, that landed him in the hospital. He endured brain surgery last year and just came back with a positive gene mutation on PCD testing and will undergo a biopsy to determine if he is just a carrier of the disease or if he is affected by PCD, an incurable lung disease, and has this marrow dysfunction. Good news is while last year he had severe pulmonary edema and inflammation, his recent CT-Scan showed no permanent damage! Nevertheless, this poor taco has endured over 15 sedated procedures/surgeries, over 10 hospital admissions, two IVIG infusions, 1 blood transfusion, 1 ICU admission, 2 Metabolic Crises, over 15 ER encounters, 1 spinal tap, two (soon to be three) bone marrow biopsies, two rounds of stomach biopsies, countless IV antibiotics, IV dextrose treatments, and others, oxygen for desaturations, weekly OT and PT therapies, feeding therapy, G-tube, NG tube, NJ tube, and over 100 blood draws.
It breaks my heart. He's so sweet. They are both so sweet and so good through all they endure. They are three and four and should just worry about playgrounds, super heroes, and cupcakes. Not spinal taps, infusions, surgeries, and biopsies. Please pray for my little bear and my big Kahuna Calvin.
INITIAL STORY AT START OF GOFUNDME:
As many of you know Calvin has had progressively worsening pulmonary and immunodeficieny issues for the past year. At first, doctors just thought it was the usual daycare illnesses with the increased difficulty of asthma. However, as the year has progressed and as Calvin's medical needs have unfolded, doctors have become increasingly concerned. It is now thought that Calvin may be suffering from a condition known as Primary Ciliary Dsykenesia along with a secondary immunodeficiency disorder. This condition, which mimics Cystic Fibrosis, makes the body unable to clear mucus and bacteria from the airways like that of a normal child making the child struggle with breathing issues, low oxygen [hypoxia], chronic cough, and frequent bacterial respiratory infections, frequently progressing to recurrent pneumonia and protracted bronchitis. Calvin has already been through two surgeries, mucus clearance therapy, and will most likely need a bronchoscopy, a much more painful procedure for ciliary biopsies [as well as less accurate] and lung cultures, especially if the noninvasive testing for Calvin cannot be done.
This noninvasive diagnostic testing for PCD, as many of you know, Calvin's insurance denied. Calvin's condition, while not curable, could be maintained successfully with an accurate diagnosis and more appropriate treatment. If it is not diagnosed accurately, however, Calvin's airways could suffer irreversible damage. Calvin is already suspected of having tracheomalacia, a type of airway damage, as a result of his illnesses and is now possibly developing bronchiectasis as well. Children who are misdiagnosed sometimes end up needing lung transplants as adults.
Calvin has a team of specialists both local and more distant. Right now he has a team of specialists at VCU that requires us to travel to Richmond on a biweekly- monthly basis for diagnostic and treatment purposes. The funds donated would go not only to the genetic testing for Calvin, but to our travel expenses as well. Any amount would help and would be so appreciated! We just want a final definitive answer for our little man so we can start the healing process for him.
Even if you cannot donate, prayers, positive thoughts, funny cat memes, and just random encouragement are always appreciated. Calvin is already lucky to be surrounded by so many wonderful family, friends, and doctors who truly love and care about him!
For more information about Primary Ciliary Dsykenesia, see:
Mr. Ted had to have a relatively minor lung procedure under anesthesia, which both boys have had multiple times. It's called a "Bronchoscopy" and involves a ciliary biopsy, numerous cultures, a "bronchoalveolar lavage," which basically means they fill the lungs with sterile water and then flush it out to "wash" and clear out any bacteria, mucus, and other things that might be in there needing to get flushed out. Most importantly, it's the most accurate way for his pulmonologist to get the overall assessment as to any improvement and/or decline in the health of his lungs and airways.
It wasn't too climatic except Teddy's lungs are still struggling despite treatment. The little guy still has mild to moderate inflammation in the lungs despite being on prophylactic antibiotics for two years now, aggressive treatment of all infections with antibiotics, no daycare/preschool, and strict adherence to thickened liquids for the last six months now. He had a little bit of bleeding in his lungs, which isn't great, but wasn't too severe. The doctor noted he seemed pretty pale, which concerned him, which likely has something to do with his platelets/marrow, but it's hard to say as his platelets were not checked.
Overall, nothing too terrible to report, but definitely no improvement and continued decline, so nothing to celebrate unfortunately. I do love Teddy (and Calvin's) Pulmonary doctor though. He truly cares about their quality of life, accurate diagnoses, and effective treatment being prompt when necessary, but not treating a child unnecessarily. He listens to parents, and just overall, truly cares about making children better and out of pain and suffering. He's a great advocate for both boys and I am so thankful for him.
Sadly, despite my requesting leave to be with Teddy while he was in the hospital, my work denied all requests, even knowing Teddy is chronically ill and was even in the hospital. I don't qualify for FMLA until Monday (when I have worked there a year), but even so, in both situations, either where: 1. I request leave and its denied or even; 2. If I had/have FMLA, I don't get paid. So, this is why the GoFundMe is so needed at times during the boys' medical journey.
Any help is appreciated, no matter how big or small. I plan to utilize the monetary donations to be by Teddy's side while he is in and out of the hospital and while he's feeling too weak or sick and needs me at home to care for him. Most importantly, the donations will allow me to not worry while I travel to St. Jude with BOTH boys, as they are thinking Calvin may actually have the same genetic mutation as Teddy (since it's inherited), and likewise, need a marrow biopsy to confirm/deny whether he's entered MDS. There is nothing more important right now then me being with Teddy to hold him, comfort him, rock him, and reassure him, while we await and (possibly) start a new treatment plan for him, and devastatingly, even Calvin. The thought that both your children; you're only children; you're most prized treasures; could both have a terminal disease is earth-shattering.
If you don't feel comfortable donating directly through GoFundMe, Facebook, or Paypal and wish to send something directly to our family, esp., the boys, and most especially little Teddy, who is going through significant hardship that no three year old should have to endure, please get in touch either by email, Facebook, or text message via my cell, for my address.
Family-wise, we could use: 1. Gas Cards to get to and from appointments as when not inpatient, we usually have Calvin's 4-5 hour infusions every three weeks and approx. three outpatient appointments in the city each week; 2. Restaurant Gift Cards for when we have to eat out after a long day of appointments; and 3. Grocery Store Gift Cards (the stores we have near us are Wegmans, Whole Foods, Trader Joes, and Instacart ((these are the only universal options).
To Keep the boys occupied at the hospital while inpatient or to use as rewards for enduring difficult procedures or testing, they adore (and can use on a hospital tray while seated): 1. LEGOS, (especially, the jurassic park, superhero, Star Wars, NexoKnights; and Ninjango sets); 2. Curious George DVDS; and 3. Hot Wheels.
I also heard from St. Jude that Calvin also will need to undergo a bone marrow failure panel and if positive, a marrow biopsy. The possibility of having my two babies- my whole world - everything I live for, having this awful disease is unimaginable. I am overwhelmed, scared, sad, and just praying for a miracle. In the event, St. Jude or Dana Farber starts treatment on Teddy after his third marrow biopsy or after going to St. Jude, I will need to take Family Leave under the Family Leave Act. While this guarantees my job back, it means I will receive NO pay while caring for my boys. Any help is appreciated as I receive no child support and absolutely will be by my boys' sides no matter what in the event they need long term treatment. Please consider giving even $5 or sharing with friends and family. Thank you.
Teddy is not doing as well. A few months back, Teddy developed a ten week long fever that doctors could not figure out. Eventually the fever was so unrelenting, spiking at times up to 104, and leaving Teddy extremely dehydrated, lethargic, and in pain, his pediatrician sent him to the Emergency Room as they suspected meningitis. He underwent an emergency spinal tap to rule out meningitis. The Emergency Room doctors suspected either an undiagnosed Primary Immunodeficiency, Infectious Disease, or Bone Marrow Failure upon his admission yet immunology and hematology both disagreed, so we waited for ID testing to return. Our amazing pulmonologist for Calvin stepped up and came immediately to Teddy's bedside & began testing for what he suspected was either Cystic Fibrosis, Primary Ciliary Dyskinesia, a Primary Immunodeficiency, or a disease called, Alpha-1 Antitrypsin.
Teddy came back with a positive pathogenic gene mutation for PCD, which means he is a carrier of the disease. However, he has to meet other clinical criteria to be confirmed with actually having the disease himself, which is still currently in process. He goes for his ciliary biopsy on August 17th, so we will be able to confirm and/or rule out PCD by September. When Teddy was discharged from inpatient, the doctor had stated his abnormal CBC, misshapen and pale blood cells, and increased atypical lymphocytes were likely due to him having Mononucleosis, however, that came back negative, so it was discouraging to have such a sick baby and no answers. By chance, I ended up meeting another mother of two children with the same rare genetic mutation (Teddy's is currently VUS) of SAMD9/SAMD9L, which predisposes one to an inherited form of pediatric bone marrow failure, which if progresses, can turn into a terminal, rapidly progressing form of leukemia, known as AML. The only cure is Bone Marrow Transplant. His Dana Farber team was not sure if Teddy's mutation would cause him to develop into marrow failure as the disease is very rare and research limited. However, after speaking with this other mother during Teddy's ongoing fevers and low blood counts, she encouraged me to reach out to St. Jude. I didn't think they would care one second about my baby, as Teddy was/is not confirmed to have cancer. Nevertheless, within hours, Teddy had a whole team of doctors at St. Jude who cared about him and started working on getting him there. He was immediately enrolled into a clinical trial at St. Jude. St. Jude requested a bone marrow biopsy, which would never have happened otherwise, as Boston was not concerned about his CBC and thought his present decline was more likely immune or pulmonary related. However, sadly, we found out that Teddy's marrow biopsy came back abnormal. The pathology report, as told to me, showed his marrow is "hypocellular," which means it is not making a sufficient amount of blood cells or as many as it should be. The pathology report also showed "dysplastic" changes, which is often the telltale sign of bone marrow failure. We won't know for sure whether or not he is or is not in a progressive state of marrow decline until we meet with St. Jude next month and have a repeat bone marrow biopsy done in a few months.
According to Teddy's local hematologist, there is a small chance Teddy's marrow transiently malfunctioned and if so, instead of progressively declining and/or developing into AML, Teddy's marrow could theoretically recover. However, other doctors of Teddy's have said these results clearly indicate he has entered marrow failure and that "dysplastic" and "hypo cellular" changes mean the marrow is in failure and he needs treatment, so it is a very difficult as a mother not knowing who is right nor knowing what Teddy's future holds in terms of his prognosis. Prior to Boston wanting to consider or discuss any treatment for pediatric MDS, due to his multi-systemic complexity, they want to be confident they know they are treating the right disease. Thus, Teddy will undergo another bone marrow biopsy in five months.
Recently, Teddy went into life-threatening metabolic crisis while in the hospital and we almost lost him, which was terrifying as this hasn't happened since he was an infant following an adrenal challenge test. They assured us they thought this would never happen again as they felt the first crisis was due to the adrenal challenge. However, after a routine MRI, Ted's glucose went from the 200s to the 30s in just minutes. Nothing is without risk, so while, I am so scared of his marrow declining further and him becoming too sick to be a transplant candidate, I appreciate his team being thoughtful prior to starting chemotherapy or prepping him for Bone Marrow Transplant as both have significant risks and thus, we need to make sure the benefits of treatment outweigh the risks and are necessary. Overall, Teddy's local hematology team said they will leave treatment decisions in St. Jude's hands and will carry out St. Jude's plan. Teddy is headed to St. Jude from September 27th-September 29th and we will learn then what the plan is - we don't know at this point. I feel blessed that Teddy was accepted in the clinical trial, as not all children with cancer or marrow failure get accepted.
I love these boys so much. I wish I did not have to watch a four and three year old endure countless IVs, surgeries, pain, fever, trouble breathing, prolonged isolation to protect from infection, hospital admissions, spinal taps, biopsies, and numerous medications, I have been by their side through every hospital admission and when home and healthy, I make each day special so we have beautiful memories. They are my whole heart, so I am praying for a miracle for Teddy. I know he is feisty and can beat this even if his repeat biopsy shows decline. Since many have asked what they can give to cheer Teddy up and/or help our family during this time, monetary donations through GoFundMe or gift card donations are the most helpful as they allow us to focus on being with Teddy while inpatient and take him to all his appointments without losing pay due to missed work. Monetary donations would also allow me to take Family Leave in the event Teddy needs long term treatment under FMLA without worrying how I will pay our bills, rent, parking, gas, and babysitter/after-school-care for Calvin so I can focus on Teddy at appointments and hospital stays. If you prefer to send gift cards - Trader Joes, Whole Foods, Wegmans, or Instacart, are great or any restaurants, especially Au Bon Pain, which is in the hospital, or gas cards. If you wish to donate something to make the boys smile during hospital procedures and stays, as many of you have requested, Target gift cards or Amazon gift cards would allow the boys to pick out prizes of their own choosing as rewards for being brave at the hospital. If you wish to purchase toys or comfort items yourself for the boys, especially Teddy, he is currently into: super heroes, dinosaurs, minions, Legos (Star Wars, Super Hero, Ninjago, or Jurassic Park themed), Play Mobile, video games for the hospital are a life savor, Hot Wheels sets, Ninjago, PJ Masks, Muppet Babies, and Darth Vader.
As for Calvin, as many of you know, Calvin's IVIG infusions were repeatedly denied in Virginia despite numerous doctors advocating and appealing on his behalf with reports of serious infections he acquired and cultured in his lungs along with failed antibiotic therapies. Well, I can't tell you what a difference it has been for Calvin to here. We had his immunological labs repeated here, as is standard at a new hospital despite having been diagnosed with his Primary Immune Deficiency in Virginia, and Calvin's numbers were still low, indicating consistency with the immune deficiency disorder he was originally diagnosed with back in Virginia. His immunologist put him on daily prophylactic antibiotics to start and gave him 6 months to see if his immune system would kick in, which he really thought it might. However, after acquiring severe pneumonia in May, his pulmonologist re-tested his immunology labs, which revealed Calvin's numbers had dropped even further. The pulmonologist and immunologist decided put Calvin on regular Immunoglobulin (Plasma) infusions this past August as a result. I just have to say as well that Calvin is SO brave and just overall such a great, great kid. He goes to Children's every 3-4 weeks for his pre-treatment regime, which is 4 medications that taste yucky, IV fluids, and then a four hour infusion and he never complains. I am so blessed and lucky to have such a sweet and perseverant little boy. All the nurses love him, as would be expected, and I have to say myself, I have never been to a hospital where EVERY nurse is so kind and caring. They have the best nurses at the infusion/transfusion center at Children's. These Plasma infusions have helped Calvin tremendously and we have seen a substantial decrease in Calvin's illnesses and episodes of respiratory distress. He has been able to stay in school like a normal kid and could not be happier. He loves being able to be at school and around friends on a consistent basis. We are so, so incredibly thankful. Unfortunately Calvin's doctors say he will require infusions for a minimum of two years and if he acquires pneumonia or any other serious infection, will likely have to increase his infusion duration to every 2 weeks. Little Calvin also had surgery this past summer due to Pulmonary and ENT needs and was inpatient for a bit, but overall he is our little fighter and is doing great on his current treatment plan.
Nevertheless, all of these successes come at a cost for our family. After work, around 4:00pm each evening, Teddy and Calvin have to go to Children's 3x each week (sometimes more) for their outpatient appointments and therapies. Each month we spend $300 or more in just parking and gas alone for the boys' outpatient appointments and therapies, which is a substantial strain on us financially. The hospital only provides the free valet parking program when your child is inpatient, so it really does add up. As most of you know, I am a single parent with a modest income. I am working full-time and even trying to work overtime on some holidays to earn more, but like most starting out in a new career, despite having my Juris Doctorate, I am still new to the work force and have to work to go up the ranks! Everyone starts somewhere though and I am so thankful to have found a job that enables the boys to receive the best treatment possible for a happy and healthy future.
Lastly, since these appointments have us getting home around 7:00pm (and on infusion nights even later around 9:00pm), we often need to eat out at the hospital, which can also add up fast. We try to pack snacks instead of buy, but when the boys are in the hospital, in order to keep their moods happy and balanced ;) , I let them pick something else if desired.
If donating monetarily is not something you are wishing to do at this time, the boys could also really use books, arts & crafts (no paint please), or other indoor activities, like LeapStart Books, to occupy them during their hospital stays, especially for Calvin's infusion days (the little guy goes through a ton of arts and crafts activities in four hours!).
Thanks for catching up with us- most of all we just ask you to keep us in your prayers and thoughts this holiday season. We are so thankful for all of you.
Kendal, you are the strongest momma I know. You and your family are always in my thoughts and prayers. Stay strong!
Prayers for your little man and your family.
Kendal - this is John Wedeles, Dana's husband. Have you looked into the pediatric aerodigestive clinic at Children's National Health System? Here is the link: http://childrensnational.org/choose-childrens/conditions-and-treatments/ear-nose-throat/aerodigestive-clinic