Calvin & Teddy's Medical Journey
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:
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.
Hey Everyone - It has been quite some time since we last did an update about our family. We needed some time to figure out the boys' medical issues, which has almost felt like starting anew from the beginning and has involved removing diagnoses, adding diagnoses, changing treatments, adding treatments, and unfortunately, adding more specialists. Between the two boys, they have to see approximately thirteen specialists at Boston Children's Hospital and Dana Farber Cancer Institute, not including just their routine pediatrician (who we adore, btw).
Since arriving here in Boston ten months ago, Teddy has required and undergone two inpatient stays, three surgeries, five outpatient procedures (two sedated), and too many appointments and lab draws to count. Teddy had brain surgery in February and was in the Pediatric Intensive Care Unit, and had to receive an emergency blood transfusion during his brain surgery. He made a full recovery though and bounced back in an incredible amount of time. In March, due to coming back with an abnormality on a bone marrow failure genetic panel, his hematologist at Dana Farber (who we also love) made the decision to regularly follow Teddy. He underwent a bone marrow biopsy in March, which thankfully revealed healthy marrow for the time being and has to go for blood-work every three months to check his marrow function. And this past summer, Teddy underwent some inpatient endocrine testing prior to his Ear, Nose, and Throat surgery and sedated bronchoscopy. Teddy started Physical Therapy and Occupational Therapy in August, at the recommendation of his neurologist, and had to be referred to orthotics to receive foot plates, and unfortunately, probably soon braces, for his walking. We do ADORE Teddy's OT and PT though and they have enabled Teddy to make so much progress, which I could not be more pleased with - I have seen Teddy achieve things I was told he would never be able to achieve!
In Virginia, I was told Teddy may not make it through brain surgery, but upon having neurosurgery here, the neurosurgeon said Teddy definitely needed the surgery as he had substantial pressure on the brain to the point part of his brain was not pulsating or receiving blood flow. In Virginia, it was suggested due to Teddy's epilepsy and neurological delays that he just remain in an infant classroom due to fall and injury risk from his epilepsy, but here, his OT and PT have him learning to climb, do stairs, build strength, and more! He is starting to be just like any other 2.5 year old, which we love! We were also told in Virginia that Teddy would likely be bedridden and on a continuous GJ feeding tube for 21 hours of his day, everyday. Luckily, the surgeon in Virginia, who also grew up with a disability, saw Teddy's potential and advocated against that and instead did a secondary GI procedure along with his G-Tube to prevent him from being on a feeding pump for the majority of his day. And since then, through much therapy, Teddy is eating ALL of his nutrition by mouth! And although he does have some ongoing swallowing issues, (like dysphasia and aspiration), and growth issues due to pancreatic insufficiency, his GI team is guardedly hopeful that he *may* get to have his G-Tube removed in the upcoming 2018 year!
I will start with the good news first. There is a lot for us to be appreciative for even in the midst of such struggle. We continue to be so blessed by an amazing community of friends and family. Due to the amazing support of everyone rooting for my two boys, as many of you know, we are officially moving to Massachusetts in mid-January. We have a place to live in Boston, my wonderful law school community has found a way to let me continue my studies while permitting Teddy to be treated at the Dana Farber Cancer Center and Boston Children's Hospital, we have new daycare set up, and Boston Children's has been amazing in coming together for Teddy's arrival. All of Teddy's appointments have been set up, he's a priority there, and the doctors are anxiously awaiting his arrival. It is so refreshing to hear doctors not afraid of treating him, optimistic, and confident in their abilities to medically care for him. Even though it's heartbreaking that my poor bear will have to re-start with approximately 12-13 doctors once we are situated in Boston, we are so blessed that he was officially accepted into their complex care program, (which is difficult to get into) and will have a specialized pediatrician in the Hospital's "Rainbow program," which provides complex care coordination and advanced expertise for children in the hospital fighting rare and life-threatening diseases. While we love a few of the doctors back home and are sad to leave them, we are confident in Teddy's new medical team and are hopeful their expertise will give him the best chance of fighting.
As for a medical update, Teddy will be inpatient this week in Maryland for biopsies and esophageal surgery. Georgetown's operating room was booked and his GI doctor felt the procedure was emergent and could not wait. He'll probably only be inpatient for a short period of time (24-48 hours) in order to stabilize him prior to the procedure with seizure medications, dextrose, and the usual IV fluids. Doctors still cannot find the overall reason why Ted has pancreatic insufficiency, (i.e., pancreatic dysfunction). In the meantime, his GI doctor is trying to treat the insufficiency with increased pancreatic enzymes to prevent further damage and promote better digestion of fats, which he is presently unable to digest, and has added two additional medications, and changed his diet. No more moo moo milk for Teddy Bear :(
As for Ted's immune system, he still cannot receive live vaccines as his doctors feel his immune system is still too weak to handle them. Their concerns about his immunodeficiencies have yet to be confirmed or refuted so it is presently too risky to administer them. We are hopeful that upon getting to Boston, however, Ted will be able to restart his plasma transfusions and eventually be strong enough to receive his remaining vaccinations without an increased risk of death or other life-threatening complications.
Neurologically, Ted is not doing well. He has temporary paralysis, collapsing, loss of swallowing and walking abilities, and is having continued seizures. His neurologist is hopeful that Ted's upcoming neurosurgery will resolve some or most of his neurological issues, if they are being caused by the increased pressure and fluid on his brain. If surgery doesn't resolve Ted's neurological issues, however, it sadly could mean he is suffering from a rare neurological-degenerative disease. And lastly, Ted's bone marrow failure testing and treatment will continue in Boston, which is great news because several of the doctors locally have not heard of the bone marrow failure conditions or rare genetic diseases Teddy is suspected of having.
As for Calvin, his pulmonologist, (who is amazing, btw), is concerned about his PCD and HA. Since Calvin is diagnosed with Primary Ciliary Dyskenesia, Common variable immune deficiency, Specific Antibody Deficiency, and Hereditary Angioedema and on a treatment plan that, (for the most part), was successful, we were able to really focus on Teddy. However, when the pulmonologist saw Calvin at his most recent visit, he noted that Calvin's cyanosis, intermittent low oxygen saturations, and spontaneous complete airway obstruction, is severe. He said the best case scenario is that Calvin has a heart defect that can be fixed surgically, but that the situation will be more severe if it's not his heart, but rather, his lungs. If his lungs are causing his episodic symptoms (not his continuous ones that are common with PCD), it means a significant portion of his lungs and airway have been damaged and cannot receive oxygen adequately. Calvin goes to the cardiologist next week to confirm the pulmonologist's suspicion that Calvin has a congenital heart defect that is causing his blood to flow in the incorrect direction. If he is correct, we are hoping the heart defect can be fixed with a simple cardiac catherization rather than an open heart surgery. :( Nevertheless, Calvin is his usual happy self, smart as a whip, so funny, and is looking forward to meeting all the princesses during his upcoming Make-A-Wish Trip!
Any donation or suggestions as to local businesses that may be willing to have a fundraising night for Teddy are appreciated so he can continue treatment at the Dana-Farber Cancer Institute are sincerely appreciated.
As for a local update, Teddy's oncologist is trying to get him into NIH so he can have better access to plasma transfusions, procedures/treatments, and genetic testing without insurance interference and we are currently awaiting Teddy's spinal MRI to see if there are any tumors or CSF buildups on his spinal cord before proceeding with neurosurgery. Now that Teddy currently has thirteen (soon to be fourteen once the neurogeneticist is added) medical specialists on his team, the medical director of the pediatric intensive care unit is kindly stepping in in attempts to assist the specialists with developing a more cohesive team-based approach to Teddy's care.
Thanks for taking the time to read Ted's update-Ted thanks everyone of you for your continued prayers, positive thoughts, and kind words.
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