Eli and Jacob are two of the most vivacious, charming, silly, and caring brothers that you will ever meet. Both boys, now ages 9 and 7, love school, reading, hanging out with friends, dancing, and spending as much time in front of a screen as their parents, Ali and Dave, will allow. They play and fight and make up, just like most siblings do.  Unfortunately, they also share the same life-threatening genetic disorder called IPEX Syndrome. IPEX causes the body’s immune system to attack its own tissues and organs, causing a variety of health problems and often affecting the intestines, skin, and endocrine system. In November of 2020 they faced their biggest challenge yet: receiving life-saving bone marrow transplants thanks to one annymous donor. 

The Children’s Organ Transplant Association (COTA) is a non-profit organization that helps children who need life-saving transplants by providing fundraising assistance and support. Click continue reading to find out more on COTA and how you can help…      

Despite their frequent illnesses and medical treatments, Eli and Jacob find joy in every day. While they are happy boys now, Eli and Jacob were both really unhappy babies. Eli got sick easily and was diagnosed early on with colic, reflux, eczema and severe food allergies.  At two months old, Jacob contracted MRSA as a result of allergy-related eczema.  His colic, chronic diarrhea, and skin issues left him constantly uncomfortable. 

In April 2015, Jacob received ear tubes and Eli’s tonsils and adenoids were removed.  As a result of Eli’s procedure, the doctors discovered that he was suffering from a pneumococcal infection that should have been prevented by routine childhood vaccinations.  This was a turning point for the Kulmans.  The doctors realized that Eli had an immune deficiency and, after testing the entire family, it turned out that Jacob did too.

Things only got worse from there. In late 2015, Eli was admitted to the hospital with a serious infection that required 5 days of IV antibiotics and another three weeks or oral antibiotics to treat. He later underwent several sinus procedures. Both boys were experiencing developmental delays becuase of their conditions but Jacob’s were more pronounced at a younger age.  Jacob had stopped eating and lost over ten percent of his body weight.  Tests showed eosinophilic disease of his GI tract.  He was admitted to the hospital, a feeding tube (G-tube) was inserted into his abdomen, and he was put on elemental formula. At just over a year old, Jacob learned how to stand and walk with the help of physical therapy and orthotics. He even practiced toddling to the nurses station holding the IV pole that was his frequent companion.  

The next few years were a blur of doctors appointments, tests, procedures, and treatments.    Both boys dealt with allergic reactions, unexplained rashes, 20+ procedures/surgeries each, PT and OT, and even more hospital admissions.  Jacob received a port to help with IVIG treatments–but the port got infected, resulting in a febrile seizure and septic infection.  He also developed alopecia and lost most of his hair. 

In May 2018, a team of experts from Children’s National Medical Center and the National Institutes of Health finally started to connect the dots to explain Eli and Jacob’s health struggles. Both boys were officially diagnosed with an exceptionally rare genetic disorder called Immunodysregulation polyendocrinopathy enteropathy X-linked (or IPEX) syndrome.  IPEX syndrome is caused by a mutation in the FOXP3 gene and is characterized by the development of multiple autoimmune disorders, often involving the intestines, skin, and endocrine glands. 

While the Kulman family finally had a name for the disease that was causing so many problems, they didn’t yet have cause to celebrate.  In a conference call with the world’s leading IPEX specialist, Ali and Dave learned that without bone marrow transplants, Eli and Jacob will likely not live past the age of 30.

In the two years since Eli and Jacob first received their diagnosis, Ali and Dave spent a lot of time researching bone marrow transplants and comparing chemotherapy and graft protocols at different hospitals.  Both boys continued to battle frequent infections and persistent rashes that limit their ability to lead normal lives, regularly attend school, and play with friends. They tried IVIG treatment for both boys, but they ended up having allergic reactions–including an anaphylactic reaction for Eli.  

In the Spring of 2019, Ali and Dave finally found a hospital and medical team they were comfortable with–even though it meant moving away from their families and loved ones.  Unfortunately, a series of setbacks led to the transplants being pushed off for another year.  Then the COVID-19 pandemic hit in March 2020 and delayed their procedures yet again.

Test results started to show an increased urgency for Eli and Jacob to receive bone marrow transplants. The combination of all of these developments led Ali and Dave to change plans.  In October the family moved temporarily to Philadelphia to be near the Children’s Hospital of Philadelphia (CHOP) and the boys started pre-transplant treatments including a brutal 10 day course of chemotherapy. On November 20th, 2020 they receieved a new lease at life with new bone marrow but this is just the start of the journey. We hope you continue to follow our blog updates and share their story. 

Eli and Jacob are strong and brave.  They have been through enough infections, illnesses, surgeries, and clinical procedures to last a lifetime–and they have many more ahead of them.  These treatments are expensive and will require follow up for the rest of their lives.  Donations to COTA in honor of Eli and Jacob will help bridge the gap between insurance coverage and actual transplant-related costs so that Ali and Dave can focus on helping the boys on their road to recovery.  

The Children’s Organ Transplant Association (COTA) helps children and young adults who need a life-saving transplant by providing fundraising assistance and family support. COTA is the nation’s only fundraising organization solely dedicated to raising life-saving dollars in honor of transplant-needy children and young adults. 100% of each contribution made to COTA in honor of our patients helps meet transplant-related expenses. COTA’s services are free to our families, and gifts to COTA are tax deductible to the fullest extent of the law.