3 Month Hospitalization

"One step forward, two steps back"

This idiomatic expression clearly captured my sentiment at the time.

We were making so much progress identifying the SVC obstruction.  The lung was scanned and biopsied in order to determine the best plan to address his cardiac lesions.  Instead, the scan revealed multiple posterior bilateral pleural nodules.  The same nodules in the heart was found in the lungs!  The lung biopsy collapsed his right lung.  Despite bilateral chest tubes, Caleb had persistent chylothorax, haemothorax, and pneumothorax during his three month hospital stay.

Emergency procedure in the ER (collapsed lung, chest tube) May 2010

After chest tube was inserted May 2010

In May 2010, not only did Caleb have a collapsed lung, the SVC obstruction was not yet addressed.  To make matters worst, the first sample from the biopsy was inconclusive.  Consequently, another biopsy had to performed to get a decent sample of the nodules.

When we finally received the results from the second biopsy, the doctors were able to place a stent in Caleb's heart.  In June 2010, after the stent, Caleb stopped his "tantrums" and he started to look better.  However, he had bilateral chest tubes, both outputting blood and chylous fluid. This lung issue continued for three months and the doctors were increasingly troubled and perplexed since the output was unstoppable.

After the stent was placed June 2010

The experts scrutinized every possible treatment plan in attempt to stop the output.  They attempted many different options such as TPN, PICC line delivery system, low fat diet, etc.  Furthermore, procedures such as pleurodesis, chest tubes, morphine drips, and blood transfusions were performed.  He also required mechanical ventilation, with settings of  breath rate of 12, PS 15, sensitivity 1 and 1-2 liters of O2.

After the pleurodesis June 2010

DIAGNOSIS

Caleb was admitted to the Pediatric Care Intensive Care Unit (PICU) in May 2010 and as of July 2010, a unifying diagnosis was still unknown.  Despite all the procedures attempted, he still required chest tubes and the ventilator.  Prognosis was not good.  At one point, a care plan was scheduled with palliative treatment on the agenda.  We were unhappy at the general tone of the conference.  It seemed the doctors were out of options and were preparing us to focus on alleviating symptoms to make Caleb comfortable and ease pain and suffering without aggressively curing the disease.

Pictures above were taken during his three month hospital stay

We were desperate.  Instead of giving up and feeling hopeless, we turned to God for guidance.  God orchestrated the next few events leading to diagnosis.  People from church were actively involved in researching possible leads.  After countless prayers, I finally had enough resources to contact.

Some of the organizations/medical facilities we contacted were:

• Cincinnati Children's Hospital
• Boston Children's Hospital/Harvard Medical School
• National Institute of Health
• Milwaukee Children's Hospital
• Lymphangiomatosis & Gorham's Disease Alliance

We sent boxes of Caleb's medical records, pictures, slides, x-rays, etc. to all who were interested in reviewing this medical conundrum.  We were persistent in making contact with providers and experts in the field.  Within 48 hours of sending the preliminary records to Boston Children's/Harvard Vascular Anomalies Center, we received the results of their multidisciplinary Vascular Anomalies conference.

Based on their findings, they believed Caleb has what they refer to as generalized lymphatic anomaly, which other institutions refer to as "lymphangiomatosis."  Caleb's imaging revealed extensive involvement in both lungs, spleen, kidneys, and mediastinum. They had recommended to continue to treat Caleb symptomatically.  They stated "while the prognosis for the disease is difficult to determine, there are some cases where patients can stabilize for a period of years but there are also those who continue to progress with worsening of their condition."
They recommended Rapamune (Sirolimus) treatment.  At the time, trials were currently underway at Cincinnati Children's Hospital for patients with lymphatic processes.   We considered participating in the clinical trial.  Due to Caleb's fragile state, we felt participation was not conducive at the time.

We prayed all the time for God to find a way to give us the medicine locally, without having to travel/participate in clinical trials.  Then in August 2010, our prayers were answered.  Several doctors advocated for Caleb to be approved for the medication and after a week, we received the medication!

After two weeks on the medication, Caleb improved.  The chest tubes were removed.  Shortly after that, we were discharged.  We were sent home with a ventilator and so many medications - mainly cardiac meds and diuretics plus the Rapamune.

Transport team getting Caleb ready to go home