The Treatment (Formula)

Kisha shipped a container of metabolic formula, Phenex, to us overnight.  She explained that we should substitute Phenex for Enfamil immediately. We were told that we'd need to bring Laurel in to the hospital again in 3 days to have her Phe levels tested.  The mix of formulas need to be adjusted depending on the results so she would receive just the right amount of phenylalanine.  This was important because too much and she would see the ill effects of PKU, too little and she would not develop properly due to lack of protein.  This is the thin line we walk daily.

Laurel rocking her shades

For the first few weeks we had to bring Laurel in for blood tests about twice a week.  This was necessary because the amount of protein she consumes, sickness, and growth rates all affect her blood phe levels.  The results of the blood tests are anything but easy to get, the blood is tested out of state so we get results based on levels from a week prior (which is aggravating sometimes).  The mix of Phenex to Enfamil jumped around quite a bit the first few weeks but eventually stabilized to a 50/50 mix.

  

Phenex

The formula is ridiculously expensive ($282.49 for 6 Cans ($3.21 / oz) on Amazon) so we are very lucky to live in a state that provides it to us.  Unfortunately, not everyone is as lucky.  Some states still do not have the necessary financial help for children with inborn errors of metabolism.  The NPKUA is fighting to get the Medical Foods Equity Act passed through congress and you should help!  It seems like a no-brainer due to the fact that the annual cost for medical formula averages $7,100 compared to the millions it would cost over time for specialized nursing care or residential care facilities to those left untreated. 

While everyone is patiently waiting for the Medical Food Equity Act to pass you should visit theFacebook page for the National PKU Food Bank (scary this is even necessary) and if you can donate medical food for those who cannot afford the formula they require, which their state/insurance will not provide.

After the initial shock of finding out Laurel had PKU we quickly fell in to a nice routine of weekly blood tests and delivering just the right amount of formula to Laurel but we knew that the challenges of “real food” were just on the horizon.